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.

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.

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.

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.

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.

Bilateral trochlear nerve palsies following dorsal midbrain haemorrhage.

Bilateral trochlear nerve palsies without other signs of dorsal midbrain syndrome following spontaneous midbrain haemorrhage is extremely rare. We report the case of a 37-year-old man with bilateral trochlear nerve palsies causing superior oblique palsies (SOP) from dorsal midbrain haemorrhage which recovered with conservative management. The report highlights the need for imaging in patients with spontaneous bilateral superior oblique (BSO) motility deficits.

Traumatic chiasmal syndrome: a series of 19 patients.

PURPOSE: To present a clinical series of 19 patients with traumatic chiasmal syndrome. METHODS: A retrospective study was performed. This included all patients with traumatic chiasmal syndrome seen in the neuro-ophthalmology clinic at the Royal Adelaide Hospital between January 1970 and January 2000. RESULTS: Of the 19 study patients, most were young males involved in motor accidents. Two-thirds had skull fractures. Three-quarters of patients had a final visual acuity of 6/12 or better in at least one eye. Ten patients had a complete optic nerve palsy. The incidence of diabetes insipidus in this study was 37%. The incidence of cranial nerve lesions, hypopituitarism, carotid cavernous fistula, and other deficits were documented. Magnetic resonance imaging and surgical findings were consistent with known mechanisms of chiasmal injury. CONCLUSIONS: Trauma is a rare cause of chiasmal syndrome. Patients with bitemporal field defects should be questioned about prior head injury. In the acute setting, magnetic resonance imaging is the most useful investigation. The treating practitioner should anticipate and treat associated endocrine, ocular motility, and other disorders. Mechanisms of damage to the optic chiasm after trauma include direct tearing, contusion haemorrhage and contusion necrosis. These mechanisms should not be considered mutually exclusive. Unilateral temporal hemianopia with a fellow blind eye is not necessarily the result of chiasmal disruption.

Optic nerve calcification after trauma.

Two patients with remote histories of severe optic nerve trauma displayed profound intraorbital optic nerve calcification on imaging studies. The presumed mechanism is optic nerve hemorrhage. Although calcification is known to occur long after brain hemorrhage, no comparable cases have been previously reported.

Craniopharyngioma: a review of long-term visual outcome.

PURPOSE: To assess the clinical presentation and long-term visual outcome in a series of patients with craniopharyn-gioma. METHODS: Retrospective case review. RESULTS: Thirty-six patients were reviewed, comprising 19 female patients and 17 male patients. The age range was 2-77 years with a bimodal distribution of 17 children (mean age 10 years) and 19 adults (mean age 47 years). Blurred vision was the most common visual complaint (23 cases, 64%) and headache the most frequent systemic complaint (19 cases, 53%). The average duration of systemic symptoms was 45 weeks compared to 10 weeks for visual symptoms. Deficits in visual acuity occurred in 13 patients (36%) and showed no significant change from initial presentation to final review. Sixteen patients (44%) had bitemporal hemianopia on presentation and pleomorphism (change from one type of visual field defect to another) occurred in 11 patients. Recurrence of tumour occurred in 15 patients (42%) and was more likely in children (59%) than adults (26%). The mean time period to recurrence was 7 years. The average follow-up period for all cases was 10 years. CONCLUSION: Patients with craniopharyngioma generally present late, and the visual symptoms are often preceded by a long history of systemic symptoms. Children are more likely to present with systemic symptoms than adults. Visual field pleomorphism is a feature of craniopharyngioma and occurred in one-third of the patients. Local recurrence is common. Although magnetic resonance imaging is the recommended means of follow up, regular neuro-ophthalmic review is useful in the early detection of anterior visual pathway compression by recurrent tumour.