Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients).

Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. Due to the rarity of this condition, diagnosis and treatment can be challenging. Diagnosis is based on the presence of the clinical triad of central nervous system dysfunction, branch retinal artery occlusions and sensorineural hearing loss. Typical MRI findings of callosal and peri-callosal lesions may assist in diagnosis. Clinical course can be monophasic, polycyclic or chronic continuous. It is important to look out for red flags to attain an accurate diagnosis and follow a therapeutic algorithm based on severity of the disease and response to treatment. Patients are treated with steroids and immunosuppressive agents with a variable response. Early aggressive treatment especially in severe cases, may help in preventing relapses and morbidity/disability. This study highlights important diagnostic features and proposes a treatment algorithm based on clinical experience from management of 16 patients from 2 neuroscience centres in the UK since 2007, who were followed up over a long period of 3-15 years.

Endonasal endoscopic management of spontaneous cerebrospinal fluid rhinorrhoea: The Birmingham UK experience.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks (SCSFL) occur in the absence of trauma, surgery, or underlying intracranial pathology. They represent a significant healthcare burden on patients with complications such as meningitis. We present our experience of SCSFL repair via the endonasal endoscopic approach. METHODS: All patients who underwent CSF fistula repair at a tertiary UK hospital, between 1st January 2012 to 31st December 2019, were identified and had their case notes analyzed retrospectively. RESULTS: There were 33 patients included consisting of 27 (81.8%) females, with age range from 31 to 81 years (mean 55.2). Mean body mass index (BMI) was 35.2 kg/m2 , with 32 (97.0%) patients overweight (BMI >25). All patients presented with intermittent watery rhinorrhoea and had a positive biochemical analysis. Computed tomography (CT) and/or magnetic resonance imaging (MRI) identified leak sites in 29 patients (87.9%). The most common intraoperative defect site was the cribriform plate (42.4%). A variety of closing techniques were used including onlay grafts (72.7%), tissue glue (87.9%), nasoseptal flaps (63.6%), mucosal free grafts (21.2%), fat grafts (21.2%), and additional support materials (87.9%). Nasal packing was used in all patients. The average length of stay was 1.6 days. Postoperative complications occurred in two patients (6.1%) (meningitis, epistaxis). Overall, there was a successful primary repair in 32 (97.0%) patients with most cases employing a multilayered reconstruction method (78.8%). CONCLUSION: Our results demonstrate excellent success rates with the endoscopic endonasal approach to SCSFL. This adds to the literature by demonstrating a detailed analysis of the experience in SCSFL management in one of the largest UK centres.

Rathke's Cleft Cyst Abscess with a Very Unusual Course.

Infected Rathke's cleft cysts (RCC) are extremely rare with only a few published cases. We report the case of a 31-year-old man who presented with headaches, visual disturbance, and hypopituitarism secondary to an infected RCC with extension of abscesses along the optic tract. Magnetic resonance imaging showed ring enhancing cystic lesions within an expanded sella with suprasellar and intraparenchymal extension. The radiological appearance suggested a high-grade optic glioma, but an endoscopic transsphenoidal biopsy revealed frank pus in the pituitary fossa, which subsequently grew Staphylococcus aureus . Pathological examination of the cyst wall showed an inflamed RCC. Following a prolonged course of intravenous antibiotics, the infection resolved and vision improved. RCC abscesses are rare and the intracranial extension of the infection in our case makes it unique.

Foster Kennedy syndrome secondary to a giant prolactinoma with a remarkable response to cabergoline.

Summary: Pituitary adenomas are intracranial neoplasms, usually demonstrating a benign phenotype. We present the case of 21-year-old male with an 18-month history of reduced visual function (acuity and field) in the left eye. Based on neuroimaging and endocrine profile, a giant prolactinoma causing hypogonadotropic hypogonadism was diagnosed and cabergoline was commenced. After a month of treatment, the tumour size reduced, and visual function improved to normal; however, he developed Foster Kennedy syndrome with a swollen right optic disc. After almost 1 year of follow-up, he regained full visual functioning. Two years since his diagnosis, his prolactin remains normal with no adverse effects or further visual complications. Learning points: Foster Kennedy syndrome is a rare entity but can be a feature of pituitary adenomas. Visual deterioration secondary to a compressive optic neuropathy can be reversible, provided that diagnosis and treatment are prompt. This case highlights the importance of frequent monitoring of visual function during follow-up of these lesions, particularly when there are deficits at diagnosis.

Metastatic Colon Cancer in the Pituitary: A Case Report with Review of Literature.

We report a rare case of metastatic colonic adenocarcinoma to the pituitary gland in a 58-year-old who presented with visual decline and panhypopituitarism. He underwent urgent transsphenoidal endoscopic surgery with significant improvement of his vision, followed by adjuvant fractionated radiotherapy to the resection cavity. He made a satisfactory recovery, but regrettably died from COVID-19 9 weeks after completion of radiotherapy. A multidisciplinary approach is essential for optimal management of this condition due to its rarity and complexity.

Acquired nystagmus.

Nystagmus is the repetitive to and fro movement of the eyes, which may be physiological or pathological. The movements can be horizontal, vertical, torsional or a combination of these movements. It starts by a slow movement of the eye away from the visual target. The second movement brings the eye back to the visual target. If this second movement is quick, the nystagmus is called jerk nystagmus. If the second movement is slow, the nystagmus is said to be pendular. Maintaining steady gaze is dependent upon visual fixation, the vestibulo-ocular reflex and the gaze-holding neural integrator system. Pathological nystagmus typically presents with the symptom of oscillopsia, which is the illusory movement of the surrounding environment. Nystagmus that develops outside of early infancy is termed acquired nystagmus. There may be serious underlying pathology that will require further investigation and management. This article reviews the terminology, pathophysiology, causes and treatment of acquired nystagmus.

Impact of the eye clinic liaison officer at an NHS foundation trust: a retrospective study.

OBJECTIVE: Vision loss has a significant impact on physical, mental and social well-being. Eye clinic liaison officers (ECLOs) have a crucial role in providing holistic care for patients with visual impairment. The aim of this study was to review the work of an ECLO over a period of 1 year at an NHS Trust to determine the volume of work and the areas of support provided by the ECLO. METHODS AND ANALYSIS: A secondary data analysis of the ECLO case notes for all patients reviewed by the ECLO at University Hospitals Birmingham (UHB) NHS Trust during the year 2019, was performed. Demographic data and certification of visual impairment (CVI) status were noted. The main outcome variable recorded was the categories of support provided by the ECLO. Case vignettes were chosen to illustrate the variety of support offered to individual patients. RESULTS: A total of 1127 consecutive participants were reviewed by the ECLO at University Hospitals Birmingham (UHB) NHS Trust during the year 2019. Areas of support most commonly provided by the ECLO included aiding the process of registration for CVI, and assistance in accessing benefits, social support and low vision support. Severely sight impaired patients required significantly more areas of support than sight impaired patients (Χ2=52.7, p=2.16×10-6). Three case vignettes, chosen by the ECLO, highlighted the positive impact of the ECLO with respect to emotional support, practical advice and as a point of contact ensuring continuity of care, also during the COVID-19 pandemic. CONCLUSIONS: The ECLO at UHB NHS Trust provides a core patient service within the ophthalmology department by being a key source of practical and emotional support and the crucial link between healthcare, social care and voluntary services.

MANAGEMENT OF ENDOCRINE DISEASE: Visual morbidity in patients with pituitary adenoma.

Visual dysfunction is an important element in the morbidity encountered in patients with pituitary adenoma leading to functional impairment and compromised quality of life. It consists of many parameters (even in the absence of reported symptomatology) as a result of tumour growth in proximity to structures critical for vision (anterior visual pathway, cranial nerves within cavernous sinuses), and as an adverse consequence of therapeutic interventions. Adenoma resection leads to high rates of visual improvement and possibly continues beyond a year post surgery, but the exact timing of maximum effect requires elucidation. Retinal nerve fibre layer measurement may be a reliable, objective parameter predicting favourable visual outcomes, although its prognostic value when pathological, needs to be confirmed. For compromised vision after pituitary apoplexy, early surgical decompression remains usual practice until evidence-based guidance becomes available. The risk of radiation-induced visual toxicity is mainly influenced by total and per fraction dose of radiation and treatment modality. Careful selection of cases and of radiotherapy technique/planning are of major importance in minimising this risk. Dopamine agonists lead to visual recovery in a considerable number of prolactinoma patients. Visual morbidity should be considered a vital indicator in the metrics of quality of service/care in pituitary disease making regular, full ophthalmic examination an essential component of modern management of pituitary pathology at all time points of patient pathway. Well-designed studies minimising effects of bias and using tools and scoring systems reliably reflecting visual status will provide robust evidence on valid prognostication and patient stratification guiding clinical decision making.

United Kingdom CSF Disorders Day 2017.

The management of patients with cerebrospinal fluid disorders requires a multi-specialty approach involving specialists in neurosurgery, neurology, neuroradiology and neuro-ophthalmology. A symposium on CSF disorders was organised by the clinical and academic teams at University Hospitals Birmingham, United Kingdom. The meeting featured a synopsis of the latest scientific research on CSF disorders. Some of the more challenging and controversial issues facing the multi-speciality team were presented and discussed. A summary of the event is provided.

Resource Planning in Glaucoma: A Tool to Evaluate Glaucoma Service Capacity.

BACKGROUND: The National Patient Safety Agency (2009) publication advising timely follow-up of patients with established glaucoma followed several reported instances of visual loss due to postponed appointments and patients lost to follow-up. The Royal College of Ophthalmologists Quality Standards Development Group stated that all hospital appointments should occur within 15% of the intended follow-up period. AIM: To determine whether: 1. Glaucoma follow-up appointments at a teaching hospital occur within the requested time 2. Appointments are requested at appropriate intervals based on the NICE Guidelines 3. The capacity of the glaucoma service is adequate Methods: A two-part audit was undertaken of 98 and 99 consecutive patients respectively attending specialist glaucoma clinics. In the first part, the reasons for delayed appointments were recorded. In the second part the requested follow-up was compared with NICE guidelines where applicable. Based on the findings, changes were implemented and a re-audit of 100 patients was carried out. RESULTS: The initial audit found that although clinical decisions regarding follow-up intervals were 100% compliant with NICE guidelines where applicable, 24% of appointments were delayed beyond 15% of the requested period, due to administrative errors and inadequate capacity, leading to significant clinical deterioration in two patients. Following the introduction of an electronic appointment tracker and increased clinical capacity created by extra clinics and clinicians, the re-audit found a marked decrease in the percentage of appointments being delayed (9%). CONCLUSIONS: This audit is a useful tool to evaluate glaucoma service provision, assist in resource planning for the service and bring about change in a non-confrontational way. It can be widely applied and adapted for use in other medical specialities.

United Kingdom CSF Disorders Day, 14th October 2016, University Hospitals Birmingham.

The management of patients with cerebrospinal fluid (CSF) disorders requires a multi-specialty approach involving specialists in neurosurgery, neurology, neuroradiology, and neuro-ophthalmology. A symposium on CSF disorders took place at University Hospitals Birmingham and covered a selection of the more challenging and controversial issues facing the multi-specialty team. The meeting also featured a synopsis of the latest scientific research. The authors provide an overview of the event.

A National Survey of Glaucoma Specialists on the Preoperative (Trabeculectomy) Management of the Ocular Surface (.).

BACKGROUND: Preserved anti-glaucoma drops cause ocular surface disease (OSD), which is increasingly being recognized as a likely cause of trabeculectomy failure. AIM: To determine the routine pre-trabeculectomy management of the ocular surface by glaucoma specialists. METHODS: A questionnaire consisting of 11 questions was posted to 146 UK glaucoma specialists. RESULTS: The first-time response rate was 43.8%. Regarding routine pre-operative management, 40.6% of specialists use preservative-free drops, 29.7% commence a drop holiday, and 53% advise lid hygiene. 42.1% prescribe lubricants, 50% prescribe topical steroids, 7.8% topical NSAIDs, and 34.4% systemic tetracyclines. 84.4% of specialists change their routine management if OSD is present. Pre-operative optimization of the ocular surface is viewed "necessary" by 48.4% and "beneficial" by 85.9%. CONCLUSION: A wide variation exists in the routine pre-operative management of the ocular surface. Research to determine the impact of different pre-operative interventions upon trabeculectomy outcomes is required.

Post-radioiodine De Novo Onset Graves' Ophthalmopathy: Case Reports and a Review of the Literature.

New-onset Graves' ophthalmopathy (GO) following radioiodine treatment (RAI) and worsening of existing GO are well-described in the endocrinology literature. These phenomena are recognized by ophthalmologists, yet poorly documented in the ophthalmology literature. Two male patients, aged 43 and 62 years, respectively, with Graves' disease without GO, received RAI. Four months later, one patient developed acute GO with unilateral reduction in visual acuity, conjunctival chemosis, lagophthalmos, bilateral severely restricted ocular motility, and lid retraction. High-dose intravenous steroids, followed by oral steroids, led to a dramatic clinical improvement. The second patient received a second dose of RAI for persistent hyperthyroidism and subsequently developed acute GO-comprising restricted ocular motility, peri-orbital swelling, and conjunctival chemosis. Symptoms gradually resolved on continued carbimazole treatment. Neither patient received pre-RAI prophylactic glucocorticoids, as currently they are only recommended for patients with pre-existing GO or multiple risk factors. We discuss the limitations of using this risk-based approach in preventing new-onset GO following RAI therapy.

Idiopathic intracranial hypertension; research progress and emerging themes.

Idiopathic intracranial hypertension (IIH) is a condition characterised by increased intracranial pressure of unknown cause predominantly seen in obese women of childbearing age and associated with a history of recent weight gain. The aetiology is poorly understood and there are no evidence-based guidelines on the management of the disease. We aim to provide a review of the recent literature outlining the latest advances in this field over the past few years. Areas of emerging interest related to the pathophysiology of IIH will be discussed, such as the role of obesity, adipose tissue and 11ß-hydroxysteroid dehydrogenase type 1. We consider the latest research on the role of venous sinus stenosis in IIH and ex vivo advances into cerebrospinal fluid drainage via the arachnoid granulation tissue. The latest techniques for optic nerve head evaluation and the role of optical coherence tomography will be summarised. Finally, we will discuss recent advances in the management of IIH, including weight loss, and medical and surgical treatment strategies.

Long-term retinoblastoma follow-up with or without general anaesthesia.

BACKGROUND: Children with treated retinoblastoma undergo frequent examinations to monitor for recurrent or new tumours. Examinations under anaesthesia allow a more complete examination in younger children, however they are stressful for the family, subject the child to medical risk and consume resources. The risk of recurrent or new tumours declines with age and it is common practice to examine older children without general anaesthesia. There are no studies on the safety and cost effectiveness of this practice, or guidelines on when examination without anaesthesia (EWA) can be safely commenced. PROCEDURE: Retrospective case note review of 128 sequential patients treated for retinoblastoma in a national referral centre over 10 years. RESULTS: Following exclusions, 113 eyes of 84 children were analysed. The mean age at diagnosis was 20 months (range birth to 71 months). There were 55 unilateral and 29 bilateral cases. The mean follow-up was 77.7 months (range 12-178 months). EWA was commenced at a mean age of 53 months (range 12-98 months). The age of conversion to EWA was largely dependent on child cooperation and disease activity. Tumour activity was detected on EWA in one child at the age of 86 months, 9 months after the last active treatment and treated successfully. CONCLUSIONS: Examination without general anaesthesia does not appear to expose children to an increased risk of undetected tumour growth. This study highlights the important factors to be considered when deciding a safe time to commence EWA.

Ocular surface disease exacerbated glaucoma: optimizing the ocular surface improves intraocular pressure control.

PURPOSE: To describe a series of 4 patients with inadequately controlled primary open angle glaucoma and ocular surface disease (OSD) in whom a combination approach was used to manage the OSD resulting in improved intraocular pressure (IOP) control. PATIENTS AND METHODS: A retrospective review of the clinical notes of 4 patients referred to a tertiary surgical glaucoma service was performed. At the initial visit, measures to control the OSD were employed in all patients; twice-daily lid hygiene measures, a 3-month course of 50 mg daily oral doxycycline, topical carmellose sodium (celluvisc) 0.5% 4 to 6 times daily, and preservative-free equivalents of topical antiglaucoma medications as deemed appropriate, depending on the perceived severity of the OSD. RESULTS: Patients were reviewed for a maximum of 24 months after intervention. In all patients treatment resulted in a marked symptomatic and clinical improvement in the ocular surface with a reduction in hyperemia, meibomian gland dysfunction and superficial keratopathy. A reduction in the IOP also occurred in all patients, obviating the need for glaucoma drainage surgery during the study period. CONCLUSIONS: Patients with severe OSD often have glaucoma that is refractive to medical therapy. Furthermore, the surgical success of glaucoma filtering surgery is compromised in patients with scarring and inflammation of the conjunctiva. The term we postulate is "OSD exacerbated glaucoma." This is the first study to suggest that the use of a combination approach comprising medical treatment to manage the OSD in patients with primary open angle glaucoma may lead to an improvement in the IOP control and the management of glaucoma.

The management of traumatic isolated inferior rectus rupture.

Traumatic inferior rectus rupture is a rare cause of diplopia following blunt trauma in the absence of blowout fracture. We report the case of a heavy goods vehicle driver, aged 42 years, with isolated rupture of the inferior rectus following blunt ocular trauma. The technique of repair, using a Hummelsheim-type procedure, is described. Good alignment was achieved at 1 month following surgery, with an excellent field of binocular vision. Other than vertical diplopia on extreme upgaze, the patient was asymptomatic and remained so 15 months following surgery. He was able to continue his job without any significant visual concerns. The surgical outcome was better than that described in previous reports of inferior rectus rupture. We feel that a Hummelsheim-type procedure is a useful option in the management of inferior rectus rupture.